Direct anticoagulant activity of protein S-C4b binding protein complex in Heerlen heterozygotes and normals

Journal of Thrombosis and Haemostasis

Volumn 2, Issue 10, 2004, Pages 1766-1773

  Heeb, M J ^a   Koenen, R R ^b   Fernandez J A ^a   Hackeng, T M ^b  

^a SCRIPPS RESEARCH INSTITUTE   (United States)

^b MAASTRICHT UNIVERSITY   (Netherlands)

Author keywords

Anticoagulant; C4b binding protein; Factor Xa; Protein S

Indexed keywords

BLOOD CLOTTING FACTOR 10A; BLOOD CLOTTING FACTOR 5A; COMPLEMENT COMPONENT C4 BINDING PROTEIN; PROTEIN C; PROTEIN S;

ANTICOAGULATION; ARTICLE; BINDING AFFINITY; BLOOD CLOTTING TIME; DISEASE ASSOCIATION; ENZYME INHIBITION; HUMAN; PRIORITY JOURNAL; PROTEIN BLOOD LEVEL; PROTEIN DETERMINATION; PROTEIN ISOLATION; SURFACE PLASMON RESONANCE; THROMBOSIS;

ADULT; ANTICOAGULANTS; BLOOD COAGULATION TESTS; BLOOD PLATELETS; FACTOR XA; FAMILY HEALTH; FEMALE; HETEROZYGOTE; HISTOCOMPATIBILITY ANTIGENS; HUMANS; MALE; PHOSPHOLIPIDS; PROTEIN BINDING; PROTEIN S; PROTHROMBIN; THROMBOPLASTIN;

EID: 13244256858     PISSN: 15387933     EISSN: None     Source Type: Journal    
DOI: 10.1111/j.1538-7836.2004.00901.x     Document Type: Article

References (35)

1. Walker FJ. Regulation of APC by a new protein. A possible function for bovine protein S. J Biol Chem 1980; 255: 5521-4.
2. Koedam JA, Meijers JCM, Sixma JJ, Bouma BN. Inactivation of FVIII by APC. Cofactor activity of protein S and protective effect of von Willebrand factor. J Clin Invest 1988; 82: 1236-43.
3. Heeb MJ, Mesters RM, Tans G, Rosing J, Griffin JH. Binding of protein S to FVa associated with inhibition of prothrombinase that is independent of APC. J Biol Chem 1993; 268: 2872-7.
4. Heeb MJ, Rosing J, Bakker HM, Fernández JA, Tans G, Griffin JH. Protein S binds to and inhibits FXa. Proc Natl Acad Sci USA 1994; 91: 2728-32.
5. Hackeng TM, van't Veer C, Meijers JCM, Bouma BN. Human protein S inhibits prothrombinase activity on endothelial cells and platelets via direct interactions with FVa and FXa. J Biol Chem 1994; 269: 21051-8.
6. Koppelman SJ, Hackeng TM, Sixma JJ, Bouma BN. Inhibition of the intrinsic FX activating complex by protein S. evidence for specific binding of protein S to FVIII. Blood 1995; 86: 1062-71.
7. van Wijnen M, Stam JG, van't Veer C, Meijers JC, Reitsma PH, Bertina RM, Bouma BN. The interaction of protein S with phospholipid surface is essential for APC-independent activity of protein S. Thromb Haemost 1996; 76: 397-403.
8. van't Veer C, Butenas S, Golden NJ, Mann KG. Regulation of prothrombinase activity by protein S. Thromb Haemost 1999; 82 80-7.
9. Comp PC, Doray D, Patton D, Esmon CT. An abnormal plasma distribution of protein S occurs in functional protein S deficiency. Blood 1986; 67: 504-8.
10. Schwarz HP, Fischer M, Hopmeier P, Batard MA, Griffin JH. Plasma protein S deficiency in familial thrombotic disease. Blood 1984; 64: 1297-300.
11. Mahasandana C, Suvatte V, Marlar RA, Manco-Johnson MJ, Jacobson LJ, Hathaway WE. Neonatal purpura fulminans associated with homozygous protein S deficiency (Letter). Lancet 1990; 335: 61-2.
12. Thommen D, Buhrfeind E, Felix R, Sulzer I, Furlan M, Lämmle B. Hämostaseparameter bei 55 Patienten mit venösen und/oder arteriellen Thromboembolien. Schweiz Med Wochenschr 1989; 119: 493-9.
13. Schwarz HP, Heeb MJ, Lottenberg R, Roberts H, Griffin JH. Familial protein S deficiency with a variant protein S molecule in plasma and platelets. Blood 1989; 74: 213-21.
14. Bertina RM, Ploos van Amstel HK, van Wijngaarden A, Coenen J, Leemhuis MP, Deutz-Terlouw PP, van der Linden IK, Reitsma PH. Heerlen polymorphism of protein S, an immunologic polymorphism due to dimorphism of residue 460. Blood 1990; 76: 533-48.
15. Duchemin J, Gandrille S, Borgel D, Feurgard P, Alhenc-Galas M, Matheron C, Dreyfus M, Dupuy E, Juhan-Vague I, Aiach M. The Ser 460 to Pro substitution of the protein S (PROS1) gene is a frequent mutation associated with free protein S (type IIa) deficiency. Blood 1995; 86: 3436-43.
16. Borgel D, Duchemin J, Alhenc-Galas M, Matheron C, Aiach M, Gandrille S. Molecular basis for protein S hereditary deficiency. Genetic defects observed in 118 patients with type I and type IIa deficiencies. The French Network on Molecular Abnormalities Responsible for Protein C and Protein S Deficiencies. J Lab Clin Med 1996; 128: 218-27.
17. Espinosa-Parrilla Y, Navarro G, Morell M, Abella E, Estivill X, Sala N. Homozygosity for the Protein S-Heerlen Allele is Associated with Type I PS Deficiency in a Thrombophilic Pedigree with Multiple Risk Factors. Thromb Haemost 2000; 83: 102-6.
18. Koenen RR, Tans G, Van Oerle R, Hamulyak K, Rosing J, Hackeng TM. The APC-independent anticoagulant activity of protein S in plasma is decreased by elevated prothrombin levels due to the prothrombin G20210A mutation. Blood 2003; 102: 1686-92.
19. Griffin JH, Gruber A, Fernández JA. Reevaluation of total, free, and bound protein S and C4b-binding protein levels in plasma anticoagulated with citrate or hirudin. Blood 1992; 79: 3203-11.
20. DiScipio RG, Hermodson MA, Yates SG, Davie EW. A comparison of prothrombin, FIX (Christmas factor), FX (Stuart factor), and protein S. Biochemistry 1977; 16: 698-706.
21. Tans G, Rosing J, Thomassen MC, Heeb MJ, Zwaal RFA, Griffin JH. Comparison of anticoagulant and procoagulant activities of stimulated platelets and platelet-derived microparticles. Blood 1991; 77 2641-8.
22. Heeb MJ, Kojima Y, Tans G, Rosing J, Griffin JH. C-terminal residues 621-635 of protein S are essential for binding to FVa. J Biol Chem 1999; 274: 36187-92.
23. Amiral J, Grosley B, Boyer-Neumann C, Marfaing-Koka A, Peynaud-Debayle E, Wolf M, Meyer D. New direct assay of free protein S antigen using two monoclonal antibodies specific for the free form. Blood Coag Fibrinol 1994; 5: 179-86.
24. Heeb MJ, Mesters RM, Griffin JH. Monoclonal antibody S4 directed against the N-terminal region of protein S blocks protein S inhibition of prothrombinase in the absence of phospholipid. Thromb Haemost 1999; (Suppl) : 565.
25. Mounier CM, Hackeng TM, Schaeffer F, Faure G, Bon C, Griffin JH. Inhibition of prothrombinase by secretory phospholipase A2 involves binding to FXa. J Biol Chem 1998; 273: 23764-72.
26. Boerger LM, Morris PC, Thurnau GP, Esmon CT, Comp PC. Oral contraceptives and gender affect protein S status. Blood 1987; 69: 692-4.
27. Schwalbe R, Dahlbäck B, Nelsestuen G. Independent association of serum amyloid P component, protein S and complement C4b with Complement C4b-binding protein. J Biol Chem 1990; 265: 21749-57.
28. Lu D, Xie RL, Rydzewski A, Long GL. The effect of N-linked glycosylation on molecular weight, thrombin cleavage, and functional activity of protein S. Thromb Haemost 1997; 77: 1156-63.
29. Rosing J, Bakker H, Thomassen MCLGD, Thomassen L, Hemker H, Tans G. Characterization of two forms of FVa with different cofactor activities. J Biol Chem 1993; 268: 21130-6.
30. Ortel TL, Quinn-Allen MA, Keller FG, Peterson JA, Larocca D, Kane WH. Localization of functionally important epitopes within the second C-type domain of FV using recombinant chimeras. J Biol Chem 1994; 269: 15898-905.
31. Fernández JA, Hackeng TM, Kojima K, Griffin JH. The carbohydrate moiety of FV modulates inactivation by APC. Blood 1997; 89: 4348-54.
32. Koenen RR, Gomes L, Tans G, Rosing J, Hackeng TM. The Ser460Pro mutation in recombinant protein S Heerlen does not affect its APC-cofactor and APC-independent anticoagulant activities. Thromb Haemost 2004; 91: 1105-14.
33. Giri TK, Yamazaki T, Sala N, Dahlback B, de Frutos PG. Deficient APC-cofactor activity of protein S Heerlen in degradation of FVa Leiden: a possible mechanism of synergism between thrombophilic risk factors. Blood 2000; 96: 523-31.
34. Morboeuf O, Borgel D, Aiach M, Kaabache T, Gandrille S, Gaussem P. Expression and characterization of recombinant protein S with Ser 460 Pro mutation. Thromb Res 2000; 100: 81-8.
35. Giri TK, Linse S, de Frutos PG, Yamazaki T, Villoutreix B, Dahlback B. Structural requirements of anticoagulant protein S for its binding to the complement regulator C4 BP. J Biol Chem 2002; 277 15099-106.